" Bilateral Ectropion in Lamellar Ichthyosis: A Rare Congenital Condition"

Abstract

Ichthyosis is a heterogenous group of dermatoses characterized by the presence of fish-like scales. The scaling is generally worse in winter. Ichthyotic disorders are usually inherited but may sometimes be acquired. Present case report describes a case of Lamellar Ichthyosis. Ichthyosis is classified into congenital and acquired types which are further subdivided as (1): A) Congenital Ichthyosis: i. Ichthyosis Vulgaris ii. X-linked Ichthyosis iii. Lamellar Ichthyosis iv. Non-bullous Ichthyosis-form Erythroderma v. Epidermolytic hyperkeratosis B) Acquired Ichthyosis I. Infections(leprosy) II. Drugs (clofazimine) III. Malignancies (lymphoma) IV. Nutritional deficiencies V. Metabolic disorders (hypothyroidism) VI. Systemic Diseases (sarcoidosis) C) Lamellar Ichthyosis Lamellar Ichthyosis is a rare congenital condition with equal distribution in both genders. At birth, child usually presents as a collodion (lacquered) baby ensheathed in a membrane; when the membrane sheds, typical scales manifest. Inheritance is Autosomal Recessive. It involves generalized body involvement which begins as collodion baby; after the membrane is shed, patient develops large thick, brown, pasted scales (fish- like) scales which persists for life. Flexures may show continuous linear rippling. Erythema is minimal; when present, it is maximum on the face. It is associated with generalized lesions, accentuated on lower extremities and flexural areas [1]. Other associated features may include • Ectropion and eclabium. • Rippled hyperkeratosis around joints which may be verrucous. • Palmer and plantar keratoderma, a frequent feature. • Crumpled ears. Molecular defect involves abnormality of gene present on chromosome 14q11, which encodes for transglutaminase [2]. Herein, we are presenting a case of Lamellar Ichthyosis associated with Bilateral Ectropion.